Primary alveolar proteinosis – A report of two cases

Bhattacharyya, D.; Barthwal,; Katoch, C.D.S.; Rohatgi, Shalesh; Hasnain, Sumaira Z.; Rai, Santosh; Arora, Arpit

doi:10.1016/j.mjafi.2012.02.016

Cited by 5 publications

(12 citation statements)

References 11 publications

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“…[1629] Among all cases, 5 (16.7%) patients were given empirical anti-tubercular therapy before diagnosis. [15203132]…”

Section: Resultsmentioning

confidence: 99%

“…No details regarding follow-up after discharge could be found for 9 studies. [1115192026273031] One study mentioned symptomatic improvement on follow-up (duration not mentioned). [20] One patient required two more sessions of WLL over during 2.5 years along with GM-CSF administration and was asymptomatic during additional 1 year of follow-up.…”

Section: Resultsmentioning

confidence: 99%

“…However, it should be noted that 5 (16.7%) patients were given empirical anti-tubercular therapy before diagnosis. [15203132] Inappropriate use of ATT is associated with adverse effects and increased risk of multidrug-resistant mycobacteria. Although this is a rare condition, physicians treating such patients should be aware of this differential diagnosis so that appropriate management may be initiated and unnecessary medications may be avoided.…”

Section: Discussionmentioning

confidence: 99%

“…[89101112131415161718192021222324252627282930313233] It should also be noted that the facility and expertise for management of PAP may not be available at all centers. We hereby report the experience of treatment of PAP from our center.…”

Section: Introductionmentioning

confidence: 99%

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Pulmonary alveolar proteinosis: Experience from a tertiary care center and systematic review of Indian literature

et al. 2016

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Background:Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by deposition of lipoproteinaceous material within alveoli, with a variable clinical course. Here, we report an experience of management of PAP at our center. A systematic review of previously reported cases from India is also included in the article.Materials and Methods:This study included patients with primary PAP managed at our center from 2009 to 2015. Diagnosis of primary PAP was based on histopathologic diagnosis on bronchoalveolar lavage or transbronchial lung biopsy and absence of causes of secondary PAP. For systematic review of Indian publications, the literature search was performed using PubMed and EMBASE databases using the terms “pulmonary alveolar proteinosis'” or “alveolar proteinosis” and “India” or “Indian.”Results:During the above-specified period, five patients with diagnosis of PAP were admitted at our center. Median age of patients was 32 years (interquartile range [IQR] 30.5–59); 80% were female. Mean duration (± standard deviation) of symptoms was 6.2 (±1.79) months. Anti-granulocyte-macrophage colony stimulating factor (GM-CSF) antibodies were elevated in 4 out of 5 patients (80%). For management, whole lung lavage (WLL) was done for four patients with median volume of 32.5 (IQR 18–74) L per patient. All the patients showed significant symptomatic as well as improvement in physiological parameters. Subcutaneous GM-CSF and ambroxol were given to 3 patients and 1 patient, respectively. The median follow-up of all patients was 18 (IQR 5–44) months. A systematic review of all Indian studies of PAP revealed thirty publications.Conclusions:WLL is the most common, effective, and safe therapy in patients with PAP. GM-CSF administration is an efficacious treatment for patients with incomplete response after WLL.

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“…[1629] Among all cases, 5 (16.7%) patients were given empirical anti-tubercular therapy before diagnosis. [15203132]…”

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pulmonary alveolar proteinosis: Experience from a tertiary care center and systematic review of Indian literature

et al. 2016

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“…[ 24 25 27 32 35 37 ] Our patient too presented with type I respiratory failure. Of the 26 patients[ 14 16 18 20 21 22 23 24 25 27 28 29 30 31 32 33 34 35 36 37 38 ] in whom CT chest was performed, HRCT was done in 23. [ 14 16 18 20 21 22 23 27 28 29 30 31 32 33 34 35 37 38 ] Characteristic “Crazy-paving” pattern was documented in 18 of them.…”

Section: Discussionmentioning

confidence: 99%

"Crazy-paving" pattern: A characteristic presentation of pulmonary alveolar proteinosis and a review of the literature from India

et al. 2016

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Diagnosing pulmonary alveolar proteinosis: Is there any role of radiology?

Sharma

Chatterjee

Debnath

2013

Medical Journal Armed Forces India

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We read with interest the article titled "Primary alveolar proteinosis e A report of two cases" written by Bhattacharyya et al 1 and published in MJAFI 2013;69:90-93. Having gone through the article, we would like to emphasize certain facts which might be relevant to all of us. The authors have presented four axial images of CT scan of chest, one photomicrograph of histopathology of open lung biopsy, one photograph depicting lung lavage fluid samples in serial order from case 1 and one axial image of CT scan of chest from case 2 in support of the case reports.The key words mentioned in the article are 1) Pulmonary alveolar proteinosis, 2) Whole lung lavage and 3) Granulocyteemacrophage colony stimulating factor. It is surprising that HRCT scan chest is not listed in the key words. As mentioned in the case history, both the cases were treated empirically with ATT despite sputum being negative for AFB and chest radiograph depicting bilateral extensive alveolar opacities. It would not be incorrect to assume at this point that the working diagnosis before the CT scan studies was pulmonary tuberculosis. Having gone through the cases mentioned in the article, it is quite evident that CT scan chest had played a decisive role in directing further investigations for confirming the diagnosis.The authors portray the HRCT findings as 'crazy paving pattern' in both the cases without any mention about the radiological diagnosis. It is on record that pulmonary alveolar proteinosis (PAP) was given as the first radiological diagnosis based on CT scan findings in both the cases despite the clinical diagnosis being pulmonary tuberculosis. It would be pertinent to mention here that radiological diagnosis of PAP was not made on the basis of 'crazy paving pattern' alone. Due consideration was given to the clinical setting in each case before suggesting the possibility of PAP. In fact, in case 2, bronchoalveolar lavage (BAL) was suggested after the CT scan study. It may also be relevant here to mention that, although 'crazy paving pattern' on HRCT chest in an appropriate setting is highly characteristic of PAP, there are a host of other conditions e.g. infectious (pneumocystis carinii pneumonia), neoplastic (mucinous bronchoalveolar carcinoma), idiopathic (sarcoidosis, non-specific interstitial pneumonia, organizing pneumonia), inhalational (lipoid pneumonia), misc (adult respiratory distress syndrome, pulmonary hemorrhage) and many more which can cause similar appearance on HRCT chest. Fig. 1 e Serial axial CT scan sections (lung window) at immediate infra-carinal (aec) and mid cardiac level (def) depicting partial clearance of lesions after nearly six months (b, e) and two years (c, f).

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Primary alveolar proteinosis – A report of two cases

Cited by 5 publications

References 11 publications

Pulmonary alveolar proteinosis: Experience from a tertiary care center and systematic review of Indian literature

Pulmonary alveolar proteinosis: Experience from a tertiary care center and systematic review of Indian literature

"Crazy-paving" pattern: A characteristic presentation of pulmonary alveolar proteinosis and a review of the literature from India

Diagnosing pulmonary alveolar proteinosis: Is there any role of radiology?

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